What is the effect of a defective a(1→ 4) phosphatase in Pompe's disease (GSD II)? O Accumulation of abnormal, unbranched glycogen, leading to heart or liver failure and short life expectancy O Glycogen accumulation in heart, leading to heart failure, muscle weakness, short life expectancy O Absence of UDP-glucose conversion to glycogen, leading to lack of muscle glycogen, exercise intolerance. Glycogen accumulation due to lack of glucose-6-phosphate conversion to glucose, leading to liver enlargement, hypoglycemia, short life oxpectancy O Lack of complete degradation of glycogen to glucose, leading to mild hypoglycemia, enlarged liver and muscle weakness Submit Request Answer
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- Understanding the Mechanism of Hemolytic Anemia Genetic defects in glycolytic enzymes can have serious consequences for humans For example, defects in the gene for pyruvate kinase can result in a condition known as hemolytic anemia. Consult a reference to learn about hemolytic anemia, and discuss why such genetic defects lead to this condition.At normal to low blood glucose concentrations (1.e., roughly 110 mg/dl or less), the enzyme that is responsible for sequestering or trapping glucose within cells is which of the following enzymes? O hexokinase O glycogen synthase O glucose-6-phosphatase O glucokinaseDifferent control mechanisms are used to regulate the synthesis of glycogen. Match the action on the left with the effect on the right. Increase in the concentration of inorganic phosphate ion Decrease in the activity of phosphoprotein phosphatase Phosphorylation of glycogen phosphorylase Dephosphorylation of glycogen synthase Ű Clear All Decreasing glycogen synthesis Increasing glycogen synthesis No effect on glycogen synthesis
- Von Gierke’s disease is also known as glycogen storage disease type I. Patients with von Gierke’s disease lackglucose 6-phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis (elevated lactate levels in the blood), especially during strenuous exercise. Explain why these symptoms occur. What chemical reaction does this enzyme catalyze? Which pathways involve this enzyme? Lacking thisenzyme will cause impairment of which pathways?• Pls consider what pathways are affected by Von Gierke’s disease. Include in your explanation involving the Cori’s cycle.Von Gierke’s disease is also known as glycogen storage disease type I. Patients with von Gierke’s disease lackglucose 6-phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis (elevated lactate levels in the blood), especially during strenuous exercise. Explain why these symptoms occur. What chemical reaction does this enzyme catalyze? Which pathways involve this enzyme? Lacking thisthe enzyme will cause impairment of which pathways?• Pls consider what pathways are affected by Von Gierke’s disease. Include in your explanation involving Cori’s cycle. can you please do not write by your hand? I mean computer if you can. thank youWhen glycogen is degraded (catabolized), all of the following occur in both liver and muscle EXCEPT Active glycogen phosphorylase catalyzes the phosphorolysis of alpha-1,4- glucosidic bonds in glycogen. The conversion of glycogen phosphorylase a to glycogen phosphorylase b is a major control step. Glucose-1-phosphate is converted to glucose-6-phosphate by phosphoglucomutase. High concentrations of glucose and AMP act as important control factors. Debranching enzyme catalyzes the hydrolysis of alpha-1,6-glucosidic bonds in glycogen.
- What is the advantage for some tumors to stop expressing the genes for glucose-6-phosphatase, phosphoenolpyruvate carboxykinase, and fructose bisphosphatase?Which enzyme activity of the glycogen debranching enzyme is operating during the release of glucose units linked to glycogen branchpoints via a1,6 glycosidic linkage? O phosphorylase activity O phosphatase activity O transferase activity O glucosidase activityWhich of the following is likely to be correct if all mutases are completely inhibited?a. Removal of glucosyl residues from nonreducing ends of glycogen in the liver is compromised;therefore, release of free glucose into blood from the liver would decreaseb. Glycerol can be efficiently converted into glucose but cannot be efficiently converted into glucosylresidues in glycogen in the liverc. Bothaandbd. Neither a nor b
- Many of the mechanisms involved in the regulation of glycogen metabolism involve covalent modification of enzymes. In particular, reversible phosphorylation/ dephosphorylation plays a central role.(a) Estimate the ΔG°′ for the dephosphorylation of phosphorylated Ser-14of glycogen phosphorylase (catalyzed by phosphoprotein phosphatase 1).Explain your reasoning.(b) Estimate the ΔG°′ for the phosphorylation of Ser-14 of glycogen phosphorylase by ATP (catalyzed by phosphorylase kinase). Explain yourreasoning.(c) Would you expect both reactions to be favorable under physiologicalconditions (i.e., to possess a negative ΔG)?Phosphoglucomutase is crucial for glycogen breakdown as well as for glycogen synthesis. Explain the role of this enzyme in each of the two processes., which indicates AG for each glycolytic reac- tion under intracellular conditions. Assume that glyceraldehyde-3- phosphate dehydrogenase was inhibited with iodoacetic acid. Which glycolytic intermediate would you expect to accumulate most rapidly, and why?